Ectopic adrenal rests (EAT) are rare events which can be incidentally found in various locations, more commonly around the adrenal glands, kidneys, or genital regions. The pathogenesis of EAT is poorly understood but probably occurs due to migration of the adrenocortical primordial cells (derived from intermediate mesoderm) with gonadal tissue. Only a few cases have been reported in other sites such as inguinal hernias, large intestine, liver, gall bladder wall, pancreas, mediastinum, and lungs.[1–6] The vast majority of these EATs being nonfunctional, they are of minimal clinical relevance and are often found as an incidental finding in other operative procedures or autopsies. However, they can give rise to hypersecretion of adrenocorticotrophic hormone causing hypertension, palpitation, etc., due to hyperplasia or even neoplasia such as pheochromocytomas, Leydig cell tumors, and adrenal adenomas.[5–7] Moreover, its recognition is important as it can pose diagnostic dilemmas due to morphological similarities with unrelated lesions.
A 21-year-old female had a right-sided ovarian cyst, clinicoradiologically suggestive of a chocolate cyst. She was operated and two separate tissues were sent for histopathological examination. The larger tissue (5.5 cm × 3.5 cm × 1 cm) was ovarian tissue and the other tissue was that of the fallopian tube (2 cm × 1.7 cm × 1 cm). Grossly, the tubal wall was congested with a tiny yellowish nodule (0.5 cm in diameter) attached to it. The ovarian tissue showed histopathological features of hemorrhagic corpus luteum and cystic follicles. No feature of an endometriotic cyst could be appreciated. The tube showed edema and vascular congestion. Section from the nodule showed encapsulated adrenocortical tissue rest composed of attenuated glomerulosa layer and predominantly zona fasciculata composed of two-cell wide packed cords of large columnar cells with distinct cell membranes, abundant, foamy cytoplasm, and uniform, pyknotic nuclei.[Figures 1 and 2]. No zona reticularis and medullary tissue were present. There was no feature of hyperplasia or neoplasia.
EAT are a form of heterotopia in which normal tissue is present in an abnormal location. It was first described as yellowish nodules around the adrenal glands by Morgagni in 1740 and since then they have been described in various sites of abdomen and pelvis. Its incidence is about 1% in adults and up to 50% in neonates in whom these rests regress within the 1st year by atrophy.[7,9,10] Since EAT arises from a defect in separation of developing adrenal cortex from the coelomic mesoderm, they can be found anywhere along the path of embryogenic migration particularly around kidneys, adrenals, retroperitoneal fat, and genitourinary organs such as ovaries, testis, spermatic cords, uterus, and broad ligaments. Unless functional, they are of minimal clinical relevance and are more commonly detected incidentally.
They can vary in size and rare cases can become hyperplastic and functional or may turn neoplastic and thus assuming large size.[11–13]
EAT is extremely rare in women with most cases arising in the broad ligament. Only a handful of cases have been reported in and around the fallopian tube.[7,11] Another entity that is more frequently encountered in the paratubal location is Walthard cell rest. Histopathologists should be aware and be able to differentiate these two entities. Walthard cell rests are benign epithelial cell clusters (may be cystic) composed of flat to cuboidal cells with elliptical nuclei and longitudinal nuclear grooves (“coffee bean” nuclei). They grossly appear as tiny yellowish or whitish nodules or cysts in the paratubal region, broad ligament or near the ovarian hilus. Walthard cell rests are often regarded as one of the sources of origin for Brenner tumors of ovary.
There is no clear-cut guideline for the management of EAT because of its rarity. Most asymptomatic, nonfunctional cases may be managed conservatively while hyperplastic, functioning, or neoplastic cases may require resection.
The recognition of this rare entity is of paramount importance to surgeons and histopathologists alike so that they can be removed during surgery and diagnosed correctly due to their potential complications.
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